Abstract IgA vasculitis, a small-vessel vasculitis characterized by IgA-dominant immune complex deposition, typically involves the skin, gastrointestinal tract, and kidneys. Although diffuse alveolar hemorrhage (DAH) is a severe manifestation of antineutrophil cytoplasmic antibody (ANCA) vasculitis, it is uncharacteristic of immune complex-mediated vasculitides. We report a rare case of DAH in the setting of IgA vasculitis. A 74-year-old man with coronary artery disease with a history of bypass surgery and type 2 diabetes presented to the emergency department with a one-month history of dyspnea and lower extremity edema. On admission, he was found to have acute kidney injury. Transthoracic echocardiography demonstrated normal biventricular systolic and diastolic function without significant valvular pathology. Right heart catheterization revealed normal right atrial, right ventricular, and pulmonary capillary wedge pressures with preserved cardiac index- indicating his acute kidney injury wasn’t secondary to heart failure or volume overload. Additional workup demonstrated hematuria and nephrotic-range proteinuria, concerning for a primary renal process. Serology including ANA, dsDNA, ANCA, anti-GBM antibody, PLA2R antibody, hepatitis panel, C3, C4, cryoglobulins, uric acid, and serum protein electrophoresis with immunofixation, was unremarkable. While awaiting renal biopsy, he developed frank hemoptysis. Chest CT demonstrated multifocal ground-glass opacities with bronchial wall thickening. Bronchoscopy revealed progressively bloodier serial bronchoalveolar lavage aliquots, consistent with DAH. Given high clinical suspicion for seronegative vasculitis, methylprednisolone 60 mg every six hours was initiated. His course was complicated by progressive respiratory and renal failure, necessitating intubation, mechanical ventilation, and continuous renal replacement therapy (CRRT). Renal biopsy revealed IgA nephropathy; however, by the time results became available, the family had elected for comfort care, and the patient had passed away. The spectrum of pulmonary involvement in vasculitis ranges from focal infiltrates to massive hemorrhage and hemoptysis. CT imaging typically shows diffuse patchy ground glass opacities and consolidations with subpleural sparing, generally with mid and lower lung zone predominance but occasionally involving upper zones. Pulmonary manifestations in IgA vasculitis are exceedingly rare, making diagnosis and management challenging. Without high-quality data in adults, treatment options are controversial. Generally, glucocorticoids are recommended as the first-line therapy and occasionally used in combination with a secondary immunosuppressive agent such as cyclosporine, tacrolimus, cyclophosphamide, and mycophenolate mofetil. Rituximab is a subject of ongoing research as it demonstrated efficacy in reported cases. Our case is notable for the occurrence of diffuse alveolar hemorrhage in IgA vasculitis, an uncommon and life-threatening presentation that underscores the need for prompt recognition and treatment. This abstract is funded by: none
Hakim et al. (Fri,) studied this question.